Severe schizencephaly without neurological abnormality

Area of Science:

• Neurology
• Developmental Neuroscience

Background:

• Schizencephaly is a rare congenital brain malformation characterized by abnormal neuronal migration.
• Epilepsy is a common comorbidity associated with various brain malformations.

Observation:

• A case of schizencephaly presenting with epilepsy is described.
• The patient exhibited normal intelligence and a largely normal neurological examination, with minor findings of flattened temporal region and anisokoria.
• Seizures began as simple partial episodes with complex hallucinations (visual, auditory, gustatory).

Findings:

• Over eight years, the patient experienced secondary generalization of seizures.
• Electroencephalogram (EEG) findings progressively worsened, correlating with increased seizure frequency.
• The patient's condition ultimately necessitated a disability pension due to intractable epilepsy.

Implications:

• This case highlights the potential for progressive and severe epilepsy in individuals with schizencephaly, even with normal intelligence.
• It underscores the importance of long-term monitoring for seizure activity and progression in schizencephaly.
• Understanding such cases can inform management strategies and prognosis for similar patients.